Congenital Heart Disease

Adult congenital heart disease should be evaluated using a systematic segmental approach. Do not assume normal connections based solely on the apparent four-chamber anatomy.

The study should define native anatomy, prior interventions, residual lesions, and their hemodynamic consequences.

Segmental Approach¶

Evaluate in sequence:

1. Cardiac position and situs
2. Systemic and pulmonary venous return
3. Atrial morphology
4. Atrioventricular connections
5. Ventricular morphology
6. Ventriculoarterial connections
7. Intracardiac shunts
8. Valves and outflow tracts
9. Great arteries
10. Chamber size and function

Ventricles should be identified by morphology rather than location.

Morphologic Right Ventricle¶

• Moderator band
• Coarse trabeculations
• More apical tricuspid valve insertion
• Tricuspid valve attaches to the septum

Morphologic Left Ventricle¶

• Fine trabeculations
• Smooth septal surface
• Elliptical shape
• Mitral valve does not attach directly to the septum

Routine Assessment¶

Document:

• Known congenital diagnosis
• Surgical and transcatheter history
• Location and patency of conduits, baffles, and shunts
• Residual or recurrent obstruction
• Residual intracardiac shunting
• Systemic and subpulmonary ventricular function
• Atrioventricular and semilunar valve disease
• Pulmonary pressure
• Aortic root and ascending aorta
• Comparison with prior congenital imaging

Use modified parasternal, apical, subcostal, and suprasternal views as necessary. Standard adult measurements may not adequately describe abnormal anatomy.

Common Adult Lesions¶

Detailed shunt assessment is reviewed in Intracardiac Shunts.

Repaired Tetralogy of Fallot¶

Evaluate:

• RV size and systolic function
• Pulmonic regurgitation
• Residual RVOT or pulmonary artery obstruction
• Residual VSD
• Tricuspid regurgitation
• Aortic root dilation
• Aortic regurgitation
• LV systolic function

CMR is preferred for accurate RV volumes and pulmonic regurgitant fraction when intervention is being considered.

Transposition and Systemic RV¶

In congenitally corrected transposition or after an atrial-switch operation, the morphologic RV supports the systemic circulation.

Evaluate:

• Systemic RV size and systolic function
• Systemic tricuspid regurgitation
• Subpulmonary LV function
• Baffle obstruction or leak
• Pulmonary venous pathway
• Outflow obstruction

Do not report ventricular identity based only on right- or left-sided position.

After an arterial-switch operation, assess:

• Neo-aortic root dilation and regurgitation
• Supravalvular pulmonary stenosis
• Branch pulmonary arteries
• Ventricular function
• Proximal coronary origins when visible

Fontan Circulation¶

A Fontan circulation lacks a subpulmonary ventricle. Systemic venous return flows passively into the pulmonary arteries.

Evaluate:

• Fontan pathway patency
• Low-velocity phasic pathway flow
• Fenestration or residual shunting
• Systemic ventricular function
• Atrioventricular valve regurgitation
• Pulmonary venous return
• Thrombus
• Pleural or pericardial effusion

Absence of high velocity does not exclude clinically important Fontan pathway obstruction. Cross-sectional imaging or catheterization may be required.

Ebstein Anomaly¶

Ebstein anomaly is characterized by apical displacement of the septal and posterior tricuspid leaflets with atrialization of the proximal RV.

Measure apical displacement from the anterior mitral annulus to the septal tricuspid leaflet insertion.

An indexed displacement >8 mm/m² supports the diagnosis.

Also assess:

• Functional RV size
• TR severity
• RA enlargement
• RV systolic function
• ASD or PFO
• RVOT obstruction

When to Escalate Imaging¶

Congenital cardiology review, TEE, CT, or CMR should be considered when:

• Anatomy or prior repair is uncertain
• Pulmonary veins are incompletely visualized
• RV size or function cannot be quantified
• A conduit or baffle is poorly seen
• A residual shunt is suspected
• Great-artery anatomy is incompletely defined
• TTE findings do not explain the clinical presentation

Suggested Reporting¶

Cardiac position and atrioventricular and ventriculoarterial connections are normal. No congenital cardiac abnormality is identified.

Findings are consistent with a secundum atrial septal defect with left-to-right flow and associated RV volume overload.

There are postoperative findings of tetralogy of Fallot repair. Severe pulmonic regurgitation is present with ___ RV dilation and ___ RV systolic function. No residual VSD is identified.

The morphologic right ventricle supports the systemic circulation and demonstrates ___ dilation with ___ systolic function. There is ___ systemic tricuspid regurgitation.

The Fontan pathway demonstrates low-velocity phasic flow without an obvious obstruction. The pathway is incompletely visualized by TTE.

Congenital anatomy and prior surgical repair are incompletely defined. Dedicated congenital imaging is recommended.

Key Points¶

• Use a segmental approach rather than relying on standard chamber position.
• Identify ventricles by morphology.
• Know the congenital diagnosis and operative history before interpreting the study.
• Report residual lesions and their hemodynamic consequences.
• CMR is preferred for RV volumes and flow quantification in many repaired lesions.
• Normal velocities do not exclude Fontan, conduit, or baffle obstruction.
• Complex congenital studies should be interpreted with congenital imaging expertise.

References¶

1. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease
2. Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale — ASE/SCAI
3. Guidelines for Performing a Comprehensive Transthoracic Echocardiographic Examination in Adults — ASE, 2019
4. Recommendations for Multimodality Assessment of Congenital Coronary Anomalies — ASE, 2020